Korean J Pathol.  1985 Sep;19(3):345-349.

Gastrointestinal Tumors Associated with von Recklinghausen's Neurofibromatosis: A report of two cases

• Park MJ
• Kim HY
• Won NH
• Kim IS
• Lee KN
• Paik SY

Abstract

Neurofibromatosis, first clearly described by von Recklinghausen in 1882, is a dominantly inherited mesodermal and ectodermal dysplasia with a broad spectrum of clinical findings. Most common is the classical neurofibromatosis, which has three major features: (1) multiple neural tumors dispersed anywhere on or in the body; (2) numerous pigmented skin lesions, some of which are "cafe au lait" spots; and (3) pigmented iris hamartomas also called Lisch nodules. Other lesions sometimes seen in patients with von Recklinghausen's disease include congenital malformations of various types, vascular lesions, neurilemoma meningioma and other intracranial neoplasms, pheochromocytoma, medullary carcinoma of thyroid gland, neuroblastoma, ganglioneuroma and Wilms' tumor. Approximately 10% of the patients with neurofibromatosis have the gastrointestinal tract tumors as neurofibroma, ganglioneuroma and leiomyoma. Some of them show evidence of malignancy. Increased activity of the protein "nerve growth factor" in the sera of the patients with disseminated neurofibromatosis has been reported. We would like to report here two cases of von Recklinghausen's neurofibromatosis with involvement of the gastrointestinal tract.

MeSH Terms

Hamartoma
Meningioma